bs_bs_banner PSYCHOGERIATRICS 2014; ••: ••–•• doi:10.1111/psyg.12064 CASE REPORT Anton–Babinski syndrome in an old patient: a case report and literature review Jiann-Jy CHEN,1 Hsin-Feng CHANG,2 Yung-Chu HSU3 and Dem-Lion CHEN4 1 Department of Neurology, Neuro-Medical Scientific Center, Buddhist Tzu Chi General Hospital, Taichung Branch, 2Faculty of Chinese Medicine, College of Chinese Medicine, China Medical University, Taichung, 3Division of Neurology, Department of Internal Medicine, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Chia-Yi, and 4G-Home Clinic, Kaohsiung, Taiwan Correspondence: Dr Yung-Chu Hsu MD, Department of Neurology, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Chia-Yi City, Taiwan. no. 539, Chung-Shao Road, Chia-Yi City 60002, Taiwan. Email: chessergg@gmail.com Received 4 November 2013; revision received 8 August 2014; accepted 15 August 2014. Key words: Anton–Babinski syndrome, blind anosognosia, cortical blindness, occipital lobe infarction, visual confabulation. Abstract Anton–Babinski syndrome is a rare disease featuring bilateral cortical blindness and anosognosia with visual confabulation, but without dementia or any memory impairment. It has a unique neuropsychiatric presentation and should be highly suspected in those with odd visual loss and imaging evidence of occipital lobe injury. In the case discussed herein, a 90-year-old man presented with bilateral blindness, obvious anosognosia, and vivid visual confabulation, which he had had for 3 days. Brain computed tomography demonstrated recent hypodense infarctions at the bilateral occipital lobes. Thus, the patient was diagnosed with Anton–Babinski syndrome. Because of his age and the thrombolytic therapy during the golden 3 hours after ischemic stroke, the patient received aspirin therapy rather than tissue plasminogen activator or warfarin. He gradually realized he was blind during the following week, but died of pneumonia 1 month later. In the literature, it is difficult to establish awareness of blindness in patients with Anton– Babinski syndrome, but optimistically, in one report, a patient was aware of blindness within 2 weeks, without vision improvement. Our case illustrates that elderly patients with Anton-Babinski syndrome can partially recover and that 1 week is the shortest time for the establishment of awareness of blindness for sufferers without vision improvement. INTRODUCTION Acute bilateral blindness is a neuro-ophthalmological emergency. In consideration of neuropsychiatric conditions in the elderly, it could be attributable to intracranial lesions (e.g. pituitary or bilateral occipital lesions),1,2 bilateral optic nerve lesions (e.g. bilateral anterior ischemic optic neuropathy caused by giant cell arteritis), and rarely, demyelinating disease or conversion disorder.3 To reach an exact localization, a detailed history and physical examinations can never be overemphasized. If a patient presents blindness and visual anosognosia, it is very difficult for the firstline physicians to make a correct diagnosis, and many assume that the condition is psychiatric, especially in elderly patients. CASE REPORT A 90-year-old man with a background of chronic obstructive pulmonary disease presented with right © 2014 The Authors Psychogeriatrics © 2014 Japanese Psychogeriatric Society hemiparesis. Brain computed tomography (CT) showed old lacunar infarctions at the bilateral basal ganglia (Fig. 1a), and magnetic resonance imaging/ angiography demonstrated an acute lacuna infarction at the left putamen and focal stenosis at the bilateral posterior cerebral arteries (Fig. 1b). The patient was treated conservatively with oral aspirin 100 mg, and the symptoms abated over the following week. He was able to lead an independent life and did so over the course of the following year. A year later, the patient presented to the emergency room because had been having frequent falls and had been unable to grasp anything put in front of him for 3 days. His initial blood pressure was 140/68 mmHg, and electrocardiography showed atrial fibrillation with a heart rate of 75 beats per minute. He was alert and stated that his vision was fine. He had poor eye contact when interviewed and could recognize his family only by sounds, but he could obey verbal 1 J-J. Chen et al. Figure 1 (a) Non-contrast brain computed tomography showed old lacunar infarctions at the bilateral basal ganglia. (b) Time-of-flight magnetic resonance angiography had shown focal stenosis (arrowheads) at the bilateral posterior cerebral arteries one year earlier. (c) Non-contrast brain computed tomography showed recent symmetric hypodensities in the bilateral occipital lobes (arrows) when Anton– Babinski syndrome occurred. orders. His muscle power and sensory function in the four limbs were full and intact. According to the ophthalmic consultation, the fundus examination was unremarkable, and the patient’s pupil size and pupillary reflexes were symmetric and intact. He did not have ptosis, ocular dysconjugate movement, excessive blinking, head wounds, or orbital swelling. Blood examinations all showed normal results. The frontline physician diagnosed him as presenting with obvious total blindness with delirium or confabulation. A neurologist was consulted to evaluate the patient’s mental status. When a pen was put in front of him, he claimed that nothing was there. He could not see the neurologist’s waving hands, and he could not obey our order to perform any pursuit, saccade, or optokinetic nystagmus test. Instead of worrying about blindness, he vividly described surroundings that did not actually exist. The three-object recall test for memory was correct. He did not have any sensory hemineglect. To exclude cortical lesions, non-contrast brain CT was performed; it demonstrated recent hypodense lesions at the bilateral occipital lobes (Fig. 1c). The patient was diagnosed with Anton–Babinski syndrome. Cardiac echo did not show significant abnormalities. Mixed-type (artherosclerotic plus cardioembolic) ischemic stroke was diagnosed, and because of his age and the thrombolytic therapy over 2 the golden 3 hours, he received aspirin 100 mg per day rather than warfarin or tissue-plasminogen activator. After education, he gradually realized that he was blind over the following week. Occupational therapy and sensory rehabilitation were recommended. However, dyspnoea gradually became exacerbated, and he could not submit to brain magnetic resonance imaging/angiography again, single-photon emission CT, or other neuropsychiatric examination. The patient died of pneumonia during the following month. DISCUSSION Anton–Babinski syndrome, also known as Anton’s syndrome, Anton’s symptom, and Anton’s blindness, was first described by Gabriel Anton in 1899.1 It was later described as blind anosognosia by Joseph François Babinski.2 In summary, Anton–Babinski syndrome is a rare neuropsychiatric syndrome featuring bilateral cortical blindness and anosognosia with visual confabulation, but without dementia or any memory impairment.3 Patients behave as if they are sighted and usually have vivid confabulation. Despite the intact anterior visual tracts, the bilateral occipital lobes are damaged. Injury to the visual association cortex accounts for the disconnection phenomenon, which explains why patients are unaware of their blindness. Visual disconnection to language areas © 2014 The Authors Psychogeriatrics © 2014 Japanese Psychogeriatric Society Anton–Babinski syndrome causes functioning speech areas to confabulate in response.3 In our case, Anton–Babinski syndrome was suspected because of blind anosognosia and visual confabulation, without dementia or any memory impairment. Also, CT confirmed new lesions at the bilateral occipital lobes (Fig. 1c), indicating bilateral cortical blindness. In addition to Anton–Babinski syndrome, other similar neuropsychiatric phenomena should also be considered when patients present with a similar condition.1 Similar neuropsychiatric phenomena include the following: akinetopsia, or motion blindness, in which patients can only see motionless objects but not moving ones;2,4 Riddoch syndrome, which manifests as residual motion vision within the hemianopic field;3,5 Charles Bonnet syndrome, which involves oriented, poor visual-acuity patients having complex visual hallucinations;4,6 Balint’s syndrome, which affects both visual perception, causing simultanagnosia and visual disorientation, and visual control of eye and hand movement, causing optic ataxia and ocular apraxia;5 prosopagnosia, a selective visual agnosia resulting from damage to a cortical network devoted to the accurate perception of faces;6,7 and Dide–Botcazo syndrome, an extension of Anton– Babinski syndrome but with involvement of memory circuitry,8 similar to the case report of temporary Anton–Babinski syndrome with amnesia.9 Our patient did not present with above conditions. Anton–Babinski syndrome is one of specific form of cortical blindness. The aetiologies include the following:1 ischemia stroke or haemorrhage involving occipital lobes;2,10–15 posterior leukoencephalopathy induced by chemotherapy,16 radiotherapy,17 preeclampsia,18 adrenoleukodystrophy,19 or mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes;3,20 obstetric haemorrhage with posterior circulation hypoperfusion;4,21 and trauma-related optic neuropathy, bifrontal contusions, and callosal disconnection.22,23 Recovery of visual function can be expected if the underlying factor is posterior reversible leukoencephalopathy or cortical hypoperfusionis, and it is corrected quickly.18,21 Ischemic stroke is the most common cause of Anton–Babinski syndrome and was the cause in our case. There have been only eight reported cases of stroke causing Anton– Babinski syndrome in the past 5 years (Table 1). The patients were aged between 51 to 96 years, and five patients were men. Only one patient’s vision recovered fully (no. 8),15 and three recovered partially (nos. 1, 2, and 7).10,11,14 The first task in rehabilitating such patients is establishing awareness of blindness.11,13 However, in the literature, it is difficult to establish awareness of blindness in patients with Anton– Babinski syndrome, but optimistically, in one report, a patient was aware of blindness within 2 weeks, without vision improvement (no. 4).12 Our patient presented was aware of his blindness within 1 week, which may be the shortest time for the establishment of awareness. Early diagnosis of Anton–Babinski syndrome enables early antiplatelet drugs therapy, which may improve vision or awareness of blindness, even in the elderly. Our patient demonstrated two hallmarks of Anton– Babinski syndrome: bilateral cortical blindness and anosognosia with visual confabulation. This builds on the limited literature on Anton–Babinski syndrome that currently exists. Furthermore, our patient had suspicious CT imaging both before and while he had Anton–Babinski syndrome; the syndrome was attributable to mixed-type (artherosclerotic plus cardioembolic) infarction due to atrial fibrillation and Table 1 Case reports of ischemia stroke with ABS, available in the English literature in the past 5 years Prognosis No Age/gender Ischemic area Insight Vision Author and case details 1 2 3 4 5 6 7 8 83/F 73/M 72/M 55/M 51/M 96/M 56/F 63/F Right O, left O-P Bilateral O, bilateral T Bilateral O Bilateral O, left P Right O Bilateral O Bilateral O Bilateral O No Not mentioned Not mentioned Yes (2 weeks) No No No Not mentioned Improved Improved Blindness Blindness Blindness Blindness Improved Recovered Maddula et al.10 Case 3 in Gaber11 Case 4 in Gaber11 Kondziella and Frahm-Falkenberg12 Eby et al.13 Carvajal et al.14 Carvajal et al.14 Spiegel et al.15 ABS, Anton–Babinski syndrome; F, female; M, male; O, occipital lobe; P, parietal lobe; T, thalamus. © 2014 The Authors Psychogeriatrics © 2014 Japanese Psychogeriatric Society 3 J-J. Chen et al. bilateral posterior cerebral arteries’ focal stenosis. Our case’s dyspnoea gradually became exacerbated so he could not submit to brain magnetic resonance imaging/angiography, brain blood flow in singlephoton emission CT, or other neuropsychiatric examination, but we observed improved visual awareness within the first week. Eventually, he died of infection so long-term follow-up was not possible. We suggest that early stroke treatment and awareness build-up are worthwhile for elderly patients with stroke-related Anton–Babinski syndrome. REFERENCES 1 Anton G. Über die Selbstwahrnehmung der Herderkrankungen des Gehirns durch den Kranken bei Rindenblindheit und Rindentaubheit. Archiv für Psychiatrie und Nervenkrankheiten 1899; 32: 86–127. (in German). 2 Babinski J. Contribution a l’étude des troubles mentaux dans l’hémiplégie organique (anosognosie). Revue Neurologique 1914; 27: 845–848. (in French). 3 Adeyemo BO, Nesathurai S. The original description of Anton syndrome. 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